vtioВестник трансплантологии и искусственных органовRussian Journal of Transplantology and Artificial Organs1995-1191Academician V.I.Shumakov National Medical Research Center of Transplantology and Artificial Organs", Ministry of Health of the Russian Federation10.15825/1995-1191-2020-2-132-138vtio-1194Research ArticleКлинические наблюденияClinical CasesТрансплантация почки с применением комплемент-блокирующей терапии у пациентки, страдающей атипичным гемолитико-уремическим синдромом, ассоциированным с антителами к фактору H: успешное предотвращение рецидива основного заболеванияKidney transplantation using complement inhibitor in a patient suffering from atypical hemolytic-uremic syndrome associated with factor H antibodies: successful prevention of recurrence of the underlying diseaseПрокопенкоЕ. И.ProkopenkoE. I.

Прокопенко Елена Ивановна

Адрес: 129110, Москва, ул. Щепкина, д. 61/2. Тел. (495) 684-57-91.

Elena Prokopenko

Address: 61/2, Shchepkina str., Moscow, 129110. Теl. (495) 684-57-91.

renalnephron@gmail.comПасовС. А.PasovS. A.ВатазинА. В.VatazinA. V.ЦалманА. Я.TsalmanA. Ya.ПанкратенкоТ. Е.PankratenkoT. E.ГенераловаГ. А.GeneralovaG. A.ГБУЗ МО «Московский областной научно-исследовательский клинический институт имени М.Ф. Владимирского»РоссияVladimirsky Moscow Regional Research Clinical InstituteRussian FederationГБУЗ МО «Московский областной научно-исследовательский клинический институт имени М.Ф. Владимирского»; ГБУЗ «Детская городская клиническая больница святого Владимира Департамента здравоохранения города Москвы»РоссияVladimirsky Moscow Regional Research Clinical Institute; St. Vladimir Children’s City Clinical HospitalRussian FederationГБУЗ «Детская городская клиническая больница святого Владимира Департамента здравоохранения города Москвы»РоссияSt. Vladimir Children’s City Clinical HospitalRussian Federation202012072020222132138Copyright © Прокопенко Е.И., Пасов С.А., Ватазин А.В., Цалман А.Я., Панкратенко Т.Е., Генералова Г.А., 20202020Прокопенко Е.И., Пасов С.А., Ватазин А.В., Цалман А.Я., Панкратенко Т.Е., Генералова Г.А.Prokopenko E.I., Pasov S.A., Vatazin A.V., Tsalman A.Y., Pankratenko T.E., Generalova G.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.transpl.ru/vtio/article/view/1194

Атипичный гемолитико-уремический синдром (аГУС) – орфанное комплемент-опосредованное заболевание, относящееся к группе тромботических микроангиопатий (ТМА), нередко рецидивирующее после трансплантации почки (ТП). Ранее ТП считалась противопоказанной и у детей, и у взрослых пациентов с аГУС из-за высокой (до 50% и выше) частоты ранних потерь трансплантатов, связанных с рецидивами ТМА после трансплантации. Введение в клиническую практику специфической комплемент-блокирующей терапии улучшило исходы у пациентов с аГУС и позволило существенно снизить риск посттрансплантационных рецидивов основного заболевания. В статье приводится описание клинического наблюдения 20-летней пациентки с аГУС, ассоциированным с антителами к основному регулятору активности комплемента – фактору H, которой была выполнена ТП с профилактическим использованием экулизумаба. В послеоперационном периоде у больной развился некроз мочеточника трансплантированной почки, потребовавший выполнения реконструктивной операции, а затем пиелонефрит трансплантата. Несмотря на послеоперационные осложнения, которые с высокой вероятностью были способны вызвать неконтролируемую активацию комплемента, рецидива ТМА удалось избежать благодаря своевременному их лечению и профилактическому применению комплемент-блокирующей терапии.

Atypical hemolytic-uremic syndrome (aHUS) is an extremely rare complement-mediated disease that belongs to the group of thrombotic microangiopathies (TMA). It often reoccurs after kidney transplantation (KT). Previously, KT was considered contraindicated in both children and adults with aHUS due to high (up to 50% and above) incidence of early graft loss associated with post-transplant recurrent TMA. Introduction of specific complement inhibitor therapy into clinical practice has improved outcomes in patients with aHUS and has significantly reduced the risk of post-transplant recurrence of underlying disease. We describe the clinical observation of a 20-year-old female patient with aHUS associated with antibodies to factor H, a major regulator of complement activation. The patient underwent KT and eculizumab was used for prophylactic purposes. In the postoperative period, the patient developed ureteral necrosis that required reconstructive surgery, followed by graft pyelonephritis. Despite postoperative complications, which were highly likely to trigger uncontrolled complement activation, TMA recurrence was avoided due to early treatment of the complications and prophylactic use of complement inhibitor therapy.

атипичный гемолитико-уремический синдромэкулизумабтрансплантация почкикомплемент-активирующие состоянияatypical hemolytic-uremic syndromeeculizumabkidney transplantationcomplement-activating conditionsАвторы выражают благодарность Е.С. Столяревич за предоставленные фотографии гистологических препаратов. Е.И. Прокопенко и Т.Е. Панкратенко участвовали в качестве лекторов образовательных программ компании «Алексион» и «Генериум». Остальные авторы заявляют об отсутствии конфликта интересов.The authorsthank Dr. E.S. Stolyarevich for histologic images. E.I. Prokopenko and Т.Е. Pankratenko had speaking engagements at educational events organized by Alexion Pharmaceuticals and Generium. The remaining authors declared no conflicts of interest.ReferencesLoirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011; 6: 60. doi: 10.1186/1750-1172-6-60.Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. 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The authors declare that there are no conflicts of interest present.